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Pénfigo
Enno Schmidt1, Michael Kasperkiewicz2, Pascal Joly3
1Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute for Experimental Dermatology, University of Lübeck, Lübeck, Germany.
Lancet (London, England)
|September 10, 2019
Resumen
El pénfigo es una enfermedad autoinmune rara que causa ampollas. El tratamiento con rituximab ofrece altas tasas de remisión y reduce los efectos secundarios de los corticosteroides en casos de moderada a grave.
Área de la Ciencia:
- Dermatología
- Inmunología
- Enfermedades autoinmunes
Sus antecedentes:
- El pénfigo es una enfermedad autoinmune grave con ampollas.
- Los autoanticuerpos patógenos se dirigen a las proteínas de adhesión desmogleína (Dsg) 1 y Dsg3.
- Esta unión de anticuerpos provoca la separación de los queratinocitos (acantólisis).
Objetivo del estudio:
- Para resumir la comprensión de la patogénesis y el diagnóstico del pénfigo.
- Para resaltar las estrategias terapéuticas actuales, incluidos los corticosteroides y el rituximab.
- Evaluar la eficacia y los beneficios de rituximab en el tratamiento del pénfigo.
Principales métodos:
- El diagnóstico implica la microscopia de inmunofluorescencia directa de las biopsias y el ELISA sérico para los anticuerpos anti-Dsg.
- El tratamiento principal incluye corticosteroides.
- El rituximab (anticuerpo anti-CD20) se utiliza para los casos moderados a severos.
Principales resultados:
- Rituximab induce una remisión completa después de la terapia en el 90% de los pacientes.
- El rituximab permite una disminución rápida de los corticosteroides.
- Esto conduce a efectos significativos de ahorro de corticosteroides y a una reducción de los eventos adversos.
Conclusiones:
- El rituximab es un tratamiento altamente eficaz para el pénfigo de moderado a severo.
- Mejora significativamente los resultados del paciente al permitir la reducción de los corticosteroides.
- El rituximab ofrece un gran beneficio en el tratamiento del pénfigo con menos efectos secundarios.
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